{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T23:42:02Z","timestamp":1780616522735,"version":"3.54.1"},"reference-count":112,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2021,2,17]],"date-time":"2021-02-17T00:00:00Z","timestamp":1613520000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI17\/01582"],"award-info":[{"award-number":["PI17\/01582"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Hereditary dominant ataxias are a heterogeneous group of neurodegenerative conditions causing cerebellar dysfunction and characterized by progressive motor incoordination. Despite many efforts put into the study of these diseases, there are no effective treatments yet. Zebrafish models are widely used to characterize neuronal disorders due to its conserved vertebrate genetics that easily support genetic edition and their optic transparency that allows observing the intact CNS and its connections. In addition, its small size and external fertilization help to develop high throughput assays of candidate drugs. Here, we discuss the contributions of zebrafish models to the study of dominant ataxias defining phenotypes, genetic function, behavior and possible treatments. In addition, we review the zebrafish models created for X-linked repeat expansion diseases X-fragile\/fragile-X tremor ataxia. Most of the models reviewed here presented neuronal damage and locomotor deficits. However, there is a generalized lack of zebrafish adult heterozygous models and there are no knock-in zebrafish models available for these diseases. The models created for dominant ataxias helped to elucidate gene function and mechanisms that cause neuronal damage. In the future, the application of new genetic edition techniques would help to develop more accurate zebrafish models of dominant ataxias.<\/jats:p>","DOI":"10.3390\/cells10020421","type":"journal-article","created":{"date-parts":[[2021,2,17]],"date-time":"2021-02-17T21:35:42Z","timestamp":1613597742000},"page":"421","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Zebrafish Models of Autosomal Dominant Ataxias"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0498-8741","authenticated-orcid":false,"given":"Ana","family":"Quelle-Regaldie","sequence":"first","affiliation":[{"name":"Department of Zoology, Genetics and Physical Anthropology, Faculty of Veterinary Science, Universidade of Santiago de Compostela, 27002 Lugo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8239-2965","authenticated-orcid":false,"given":"Daniel","family":"Sobrido-Came\u00e1n","sequence":"additional","affiliation":[{"name":"Department of Functional Biology, CIBUS, Faculty of Biology, Universidade de Santiago de Compostela, 15782 Santiago de Compostela, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7507-080X","authenticated-orcid":false,"given":"Ant\u00f3n","family":"Barreiro-Iglesias","sequence":"additional","affiliation":[{"name":"Department of Functional Biology, CIBUS, Faculty of Biology, Universidade de Santiago de Compostela, 15782 Santiago de Compostela, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Jes\u00fas","family":"Sobrido","sequence":"additional","affiliation":[{"name":"Instituto de Investigaci\u00f3n Biom\u00e9dica de A Coru\u00f1a (INIBIC), Servicio Galego de Sa\u00fade, 15006 Coru\u00f1a, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7927-5303","authenticated-orcid":false,"given":"Laura","family":"S\u00e1nchez","sequence":"additional","affiliation":[{"name":"Department of Zoology, Genetics and Physical Anthropology, Faculty of Veterinary Science, Universidade of Santiago de Compostela, 27002 Lugo, Spain"},{"name":"Preclinical Animal Models Group, Health Research Institute of Santiago de Compostela (IDIS), 15706 Santiago de Compostela, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1136\/jnnp-2014-308421","article-title":"The Autosomal Dominant Spinocerebellar Ataxias: Emerging Mechanistic Themes Suggest Pervasive Purkinje Cell Vulnerability","volume":"86","author":"Hekman","year":"2015","journal-title":"J. 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